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Childhood hearing loss

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https://www.readbyqxmd.com/read/28188436/clinical-and-molecular-aspects-of-distal-renal-tubular-acidosis-in-children
#1
Martine T P Besouw, Marc Bienias, Patrick Walsh, Robert Kleta, William G Van't Hoff, Emma Ashton, Lucy Jenkins, Detlef Bockenhauer
BACKGROUND: Distal renal tubular acidosis (dRTA) is characterized by hyperchloraemic metabolic acidosis, hypokalaemia, hypercalciuria and nephrocalcinosis. It is due to reduced urinary acidification by the α-intercalated cells in the collecting duct and can be caused by mutations in genes that encode subunits of the vacuolar H(+)-ATPase (ATP6V1B1, ATP6V0A4) or the anion exchanger 1 (SLC4A1). Treatment with alkali is the mainstay of therapy. METHODS: This study is an analysis of clinical data from a long-term follow-up of 24 children with dRTA in a single centre, including a genetic analysis...
February 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28167020/early-childhood-otitis-media-and-later-school-performance-a-prospective-cohort-study-of-associations
#2
Vincent Fougner, Asbjørn Kørvel-Hanquist, Anders Koch, Jesper Dammeyer, Janni Niclasen, Jørgen Lous, Preben Homøe
INTRODUCTION: Otitis media (OM) is a common disease in childhood and hearing loss (HL) is the most common complication. Prolonged HL may lead to language delay and cognitive difficulties. However, the consequences of HL due to OM are not fully understood. The aim of this study was to determine the possible association between number of OM episodes in childhood and self-rated school performance controlling for potential confounders. METHODS: Prospectively gathered systematic interview data on OM episodes in early childhood and school performance at 11 years of age were obtained from The Danish National Birth Cohort, involving >100,000 individual pregnancies and their offspring...
March 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28167017/hearing-sensitivity-in-adults-with-a-unilateral-cleft-lip-and-palate-after-two-stage-palatoplasty
#3
I F P M Kappen, J B S Schreinemakers, K P Q Oomen, D Bittermann, M Kon, C C Breugem, A B Mink van der Molen
OBJECTIVE: To evaluate long-term hearing and middle ear status in patients treated for a unilateral complete cleft lip and palate (UCLP) by two-stage palatoplasty. METHODS: Forty-nine UCLP patients aged 17 years and older were included in this retrospective study. Patients were invited for a multidisciplinary long-term follow-up of their treatment at a tertiary center for craniofacial surgery in the Netherlands. ENT assessment included tympanometry and pure-tone audiometry...
March 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28167001/childhood-hearing-loss-a-need-for-primary-health-care
#4
Segun Ayodeji Ogunkeyede, Stephen Oluwatosin Adebola, Abdulwasiu Salman, Akeem Olawale Lasisi
INTRODUCTION: Essential health care for children is the care of the ear. METHODS: A cross-sectional descriptive study of 155 children with hearing loss. RESULTS: A total of 155 pupils with hearing impairment and their parents were interviewed; 77(49.7%) males and 78(50.3%) females, age ranged from 6 to 15years (mean 9.11 ± 2.5 years). None of the participants had neonatal hearing screening. Parents detected the hearing loss at a mean age of 2...
March 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28100557/dual-sensory-impairment-in-special-schools-in-south-eastern-nigeria
#5
Ada E Aghaji, Richard Bowman, Vincent C Ofoegbu, Andrew Smith
In a cross-sectional study to determine the magnitude of dual sensory impairment (DSI-combined hearing and vision loss) in children in single-disability special education schools, children in schools for the blind and schools for the deaf in four states in South-East Nigeria were examined by an ophthalmologist and otorhinolaryngologist to determine the level of their disability and to identify other disabilities if any. Participants were all students with childhood blindness or childhood deafness. The magnitude and causes of DSI and the burden of undetected DSI were the main outcome measures...
February 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28088980/indicators-for-continuous-quality-improvement-for-otitis-media-in-primary-health-care-for-aboriginal-and-torres-strait-islander-children
#6
Beverly Sibthorpe, Jason Agostino, Harvey Coates, Sharon Weeks, Deborah Lehmann, Marianne Wood, Francis Lannigan, Daniel McAullay
Otitis media is a common, generally self-limiting childhood illness that can progress to severe disease and have lifelong sequelae, including hearing loss and developmental delays. Severe disease is disproportionately prevalent among Aboriginal and Torres Strait Islander children. Primary health care is at the frontline of appropriate prevention and treatment. Continuous quality improvement in the prevention and management of important causes of morbidity in client populations is accepted best practice in primary health care and now a requirement of Australian Government funding to services providing care for Aboriginal and Torres Strait Islander children...
January 16, 2017: Australian Journal of Primary Health
https://www.readbyqxmd.com/read/28067750/unilateral-hearing-loss-understanding-speech-recognition-and-localization-variability-implications-for-cochlear-implant-candidacy
#7
Jill B Firszt, Ruth M Reeder, Laura K Holden
OBJECTIVES: At a minimum, unilateral hearing loss (UHL) impairs sound localization ability and understanding speech in noisy environments, particularly if the loss is severe to profound. Accompanying the numerous negative consequences of UHL is considerable unexplained individual variability in the magnitude of its effects. Identification of covariables that affect outcome and contribute to variability in UHLs could augment counseling, treatment options, and rehabilitation. Cochlear implantation as a treatment for UHL is on the rise yet little is known about factors that could impact performance or whether there is a group at risk for poor cochlear implant outcomes when hearing is near-normal in one ear...
November 4, 2016: Ear and Hearing
https://www.readbyqxmd.com/read/28049114/a-targeted-approach-for-congenital-cytomegalovirus-screening-within-newborn-hearing-screening
#8
Karen B Fowler, Faye P McCollister, Diane L Sabo, Angela G Shoup, Kris E Owen, Julie L Woodruff, Edith Cox, Lisa S Mohamed, Daniel I Choo, Suresh B Boppana
BACKGROUND AND OBJECTIVE: Congenital cytomegalovirus (cCMV) infection remains a leading cause of childhood hearing loss. Currently universal CMV screening at birth does not exist in the United States. An alternative approach could be testing infants who do not pass their newborn hearing screening (NHS) for cCMV. This study was undertaken to evaluate whether a targeted approach will identify infants with CMV-related sensorineural hearing loss (SNHL). METHODS: Infants born at 7 US medical centers received NHS and were also screened for cCMV while in the newborn nursery...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28043400/dental-issues-in-lacrimo-auriculo-dento-digital-syndrome-an-autosomal-dominant-condition-with-clinical-and-genetic-variability
#9
M J Hajianpour, Hannah Bombei, Scott M Lieberman, Rachael Revell, Rachana Krishna, Robert Gregorsok, Simon Kao, Jeff M Milunsky
BACKGROUND AND OVERVIEW: Lacrimo-auriculo-dento-digital (LADD) syndrome is an autosomal dominant disorder with variable lacrimal and salivary gland hypoplasia and aplasia, auricular anomalies and hearing loss, dental defects and caries, and digital anomalies. CASE DESCRIPTION: The authors present the cases of 2 unrelated children with enamel defects and history of dry mouth leading to recurrent dental caries. The referring diagnoses were Sjögren disease and hypohidrotic ectodermal dysplasia, respectively...
December 30, 2016: Journal of the American Dental Association
https://www.readbyqxmd.com/read/28039368/long-term-neurologic-health-and-psychosocial-function-of-adult-survivors-of-childhood-medulloblastoma-pnet-a-report-from-the-childhood-cancer-survivor-study
#10
Allison A King, Kristy Seidel, Chongzhi Di, Wendy M Leisenring, Stephanie Mabry Perkins, Kevin R Krull, Charles A Sklar, Daniel M Green, Gregory T Armstrong, Lonnie K Zeltzer, Elizabeth Wells, Marilyn Stovall, Nicole J Ullrich, Kevin C Oeffinger, Leslie L Robison, Roger J Packer
BACKGROUND: Medulloblastoma is the most common malignant childhood brain tumor, although long-term risks for chronic neurologic health and psychosocial functioning in aging adult survivors are incompletely characterized. METHODS: The Childhood Cancer Survivor Study (CCSS) includes 380 five-year survivors of medulloblastoma/primitive neuroectodermal tumor (PNET; median age at follow-up: 30 y, interquartile range 24-36) and sibling comparison (n = 4031). Cumulative incidence of neurologic health conditions was reported...
December 29, 2016: Neuro-oncology
https://www.readbyqxmd.com/read/28012515/the-utility-of-early-developmental-assessments-on-understanding-later-nonverbal-iq-in-children-who-are-deaf-or-hard-of-hearing
#11
Jareen Meinzen-Derr, Susan Wiley, Jannel Phillips, Mekibib Altaye, Daniel I Choo
OBJECTIVE: In children who are deaf or hard of hearing (DHH), it is helpful to have meaningful early measures of development in order to provide effective interventions and offer benchmarks that help recognize varied developmental trajectories. The main objective of this study was to compare results of an early developmental assessment prior to 3 years of age to later nonverbal IQ assessed between 3 and 6 years of age in children who are DHH. METHODS: This study included children 3-6 years of age with bilateral permanent hearing who were enrolled in a prospective cohort study on developmental outcomes...
January 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/27958602/severe-to-profound-deafness-may-be-associated-with-myh9-related-disease-report-of-4-patients
#12
P Canzi, A Pecci, M Manfrin, E Rebecchi, C Zaninetti, V Bozzi, M Benazzo
MYH9-related disease (MYH9-RD) is a rare genetic syndromic disorder characterised by congenital thrombocytopenia and is associated with the risk of developing progressive sensorineural hearing loss, nephropathy and presenile cataracts during childhood or adult life. All consecutive patients enrolled in the Italian Registry for MYH9-RD with severe to profound deafness were included in a retrospective study. The study population involved 147 Italian patients with MYH9-RD: hearing loss was identified in 52% of cases and only 4 patients (6%) presented severe to profound deafness at a mean age of 33 years...
October 2016: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/27924730/congenital-malformations-attributed-to-prenatal-exposure-to-cyclophosphamide
#13
Padmanabhan Rengasamy
Cyclophosphamide (CPA) remains one of the most widely prescribed anticancer drugs. It is also used in the treatment of rheumatoid arthritis, childhood nephrotic syndrome and systemic lupus erythematosus. It is a potent immunosuppressive agent. It is commonly used in blood and bone marrow transplantation. With the growing trend among women postponing childbearing, the number of women who are diagnosed with breast cancer is also increasing thus escalating the chances of exposure of the unborn child to antineoplastic drugs...
December 6, 2016: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/27918236/meniere-s-migraine-and-motion-sickness
#14
John F Golding, Mitesh Patel
CONCLUSION: Elevated Motion Sickness Susceptibility (MSS) in Meniere?s disease (MD) is likely to be a consequence of the onset of MD and not migraine per se. OBJECTIVES: Pathologies of the vestibular system influence MSS. Bilateral vestibular deficits lower MSS, vestibular neuritis or benign paroxysmal positional vertigo have little overall effect, whereas vestibular migraine elevates MSS. However, less is known about MSS in MD, a condition in which many patients experience vestibular loss and migraine symptoms...
December 5, 2016: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/27896131/case-report-5%C3%A2-year-follow-up-of-adult-late-onset-mitochondrial-encephalomyopathy-with-lactic-acid-and-stroke-like-episodes-melas
#15
Kiri Sunde, Patrick R Blackburn, Anvir Cheema, Jennifer Gass, Jessica Jackson, Sarah Macklin, Paldeep S Atwal
Mitochondrial encephalomyopathy with lactic acid and stroke-like episodes (MELAS) is a multisystem mitochondrial disorder that typically presents in childhood. We describe the follow-up of a patient who was diagnosed with late-onset MELAS at the age of 49. Her clinical course includes sensorineural hearing loss, seizures, and multiple episodes of stroke-like metabolic crises. Molecular genetic testing on whole blood revealed 31% heteroplasmy of a m.3243A > G variant in the mtDNA, the causative variant in approximately 80% of MELAS cases...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27776808/evaluation-of-family-history-of-permanent-hearing-loss-in-childhood-as-a-risk-indicator-in-universal-screening
#16
Mercedes Valido Quintana, Ángeles Oviedo Santos, Silvia Borkoski Barreiro, Alfredo Santana Rodríguez, Ángel Ramos Macías
INTRODUCTION AND OBJECTIVE: Sixty percent of prelingual hearing loss is of genetic origin. A family history of permanent childhood hearing loss is a risk factor. The objective of the study is to determine the relationship between this risk factor and hearing loss. We have evaluated clinical and epidemiological characteristics and related nonsyndromic genetic variation. MATERIAL AND METHOD: This was a retrospective, descriptive and observational study of newborns between January 2007 and December 2010 with family history as risk factor for hearing loss using transient evoked otoacoustic emissions and auditory brainstem response...
October 21, 2016: Acta Otorrinolaringológica Española
https://www.readbyqxmd.com/read/27771768/advances-in-genetic-hearing-loss-cib2-gene
#17
Agnieszka Jacoszek, Agnieszka Pollak, Rafał Płoski, Monika Ołdak
Hearing plays a crucial role in human development. Receiving and processing sounds are essential for the advancement of the speech ability during the early childhood and for a proper functioning in the society. Hearing loss is one of the most frequent disabilities that affect human senses. It can be caused by genetic or environmental factors or both of them. Calcium- and integrin-binding protein 2 (CIB2) is one of the recently identified genes, involved in HI pathogenesis. CIB2 is widely expressed in various human and animal tissues, mainly in skeletal muscle, nervous tissue, inner ear, and retina...
October 22, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27761875/saving-deaf-children-screening-for-hearing-loss-as-a-public-interest-case
#18
Sigrid Bosteels, Michel Vandenbroeck, Geert Van Hove
New-born screening programs for congenital disorders and chronic disease are expanding worldwide and children "at risk" are identified by nationwide tracking systems at the earliest possible stage. These practices are never neutral and raise important social and ethical questions. An emergent concern is that a reflexive professionalism should interrogate the ever earlier interference in children's lives. The Flemish community of Belgium was among the first to generalize the screening for hearing loss in young children and is an interesting case to study the public justification of early interventions for families with deaf children...
October 19, 2016: Journal of Bioethical Inquiry
https://www.readbyqxmd.com/read/27746216/early-age-noise-exposure-increases-loudness-perception-a-novel-animal-model-of-hyperacusis
#19
Ana'am Alkharabsheh, Fen Xiong, Senthilvelan Manohar, Guangdi Chen, Richard Salvi, Wei Sun
The neural mechanisms that give rise to hyperacusis, a reduction in loudness tolerance, are largely unknown. Some reports suggest that hyperacusis is linked to childhood hearing loss. However, the evidence for this is largely circumstantial. In order to rigorously test this hypothesis, we studied loudness changes in rats caused by intense noise exposure (12 kHz narrow band noise, 115 dB SPL, 4 h) at postnatal 16 days. Rats without noise exposure were used as controls. The exposed noise group (n = 7) showed a mean 40-50 dB hearing loss compared to the control group (n = 8) at high frequencies (>= 8 kHz) and less hearing loss at lower frequencies...
October 13, 2016: Hearing Research
https://www.readbyqxmd.com/read/27729161/associations-between-childhood-hearing-loss-and-behavioural-and-academic-difficulties-a-danish-cohort-study
#20
Janni Niclasen, Carsten Obel, Christian Guldager, Simone Pleinert, Jesper Dammeyer
OBJECTIVE: Negative associations between hearing loss (HL) and behavioural and academic difficulties have been reported. However, most studies are based on small clinical samples. The aim of the present study was to investigate such associations using data from a large-scale non-clinical Danish birth cohort controlling for a large number of relevant confounding factors. METHODS: The study applied data from the Aarhus Birth Cohort's 10-12-year-old follow-up (N = 7599)...
November 2016: International Journal of Pediatric Otorhinolaryngology
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